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Focal cortical dysplasia type IIIc

MedGen UID:
1671107
•Concept ID:
C4732828
•
Disease or Syndrome
HPO: HP:0032057

Definition

A subtype of focal cortical dysplasia type III that is characterized by alterations in architectural (cortical dyslamination, hypoplasia) or cytoarchitectural composition of the neocortex (hypertrophic neurons), which occur adjacent to vascular malformations (cavernomas, arteriovenous malformations, leptomeningeal vascular malformations, telangiectasias, meningioangiomatosis). [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVFocal cortical dysplasia type IIIc

Professional guidelines

PubMed

Neuropathological work-up of focal cortical dysplasias using the new ILAE consensus classification system - practical guideline article invited by the Euro-CNS Research Committee.
Blümcke I, Mühlebner A
Clin Neuropathol 2011 Jul-Aug;30(4):164-77. doi: 10.5414/np300398. PMID: 21726501

Recent clinical studies

Etiology

Variable histopathology features of neuronal dyslamination in the cerebral neocortex adjacent to epilepsy-associated vascular malformations suggest complex pathogenesis of focal cortical dysplasia ILAE type IIIc.
Miyata H, Kuwashige H, Hori T, Kubota Y, Pieper T, Coras R, Blümcke I, Yoshida Y
Brain Pathol 2022 Sep;32(5):e13052. Epub 2022 Jan 9 doi: 10.1111/bpa.13052. PMID: 35001442Free PMC Article
ILAE focal cortical dysplasia type IIIc in the ictal onset zone in epileptic patients with solitary meningioangiomatosis.
Mukae N, Suzuki SO, Morioka T, Murakami N, Hashiguchi K, Shigeto H, Sakata A, Iihara K
Epileptic Disord 2014 Dec;16(4):533-9. doi: 10.1684/epd.2014.0695. PMID: 25366924
Encephalomalacic dysplastic mass lesion associated with vascular abnormalities in an elderly man. Is this "acquired" focal cortical dysplasia?
Takei H, Bhattacharjee MB
Int J Clin Exp Pathol 2013;6(10):2267-71. Epub 2013 Sep 15 PMID: 24133610Free PMC Article

Diagnosis

ILAE focal cortical dysplasia type IIIc in the ictal onset zone in epileptic patients with solitary meningioangiomatosis.
Mukae N, Suzuki SO, Morioka T, Murakami N, Hashiguchi K, Shigeto H, Sakata A, Iihara K
Epileptic Disord 2014 Dec;16(4):533-9. doi: 10.1684/epd.2014.0695. PMID: 25366924

Clinical prediction guides

Variable histopathology features of neuronal dyslamination in the cerebral neocortex adjacent to epilepsy-associated vascular malformations suggest complex pathogenesis of focal cortical dysplasia ILAE type IIIc.
Miyata H, Kuwashige H, Hori T, Kubota Y, Pieper T, Coras R, Blümcke I, Yoshida Y
Brain Pathol 2022 Sep;32(5):e13052. Epub 2022 Jan 9 doi: 10.1111/bpa.13052. PMID: 35001442Free PMC Article

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